Bleeding Disorders Flashcards
Which blood components are most likely to be responsible for bleeding disorders?
1 - Platelets
2 - Von Willebrand Factor
3 - Coagulation factors
What are important points that should be established in a bleeding disorder history taking?
1 - Do they actually have a bleeding disorder
2 - How severe is the disorder
3 - What is the pattern of bleeding
4 - Is the condition congenital or acquired
5 - What is the mode of inheritance
What are common features that are present when taking a history from a patient with a bleeding disorder?
1 - Easily bruised
2 - Epistaxis
3 - Post-surgical bleeding
4 - Menorrhagia
5 - Post-partum haemorrhage
6 - Post-trauma bleeding
When considering the severity of a bleeding disorder, what needs to be considered?
How appropriate is the bleeding? I.e. bleeing from being stabbed in the chest (very appropriate) compared to bleeding standing up straight (very inappropriate)
What is the pattern of bleeding associated with platelets and VWF?
They bleed onto surfaces (mucosa, menorrhagia, GI)
image - non-blanching petechiae
What is the bleeding pattern associated with coagulation factors?
They bleed into spaces (hinge joints, muscles, CNS)
What type of inheritance does Haemophilia A and B have?
X-linked recessive
What does a X-linked recessive transmission indicate about the likely disease status of the parents?
A father with the defective gene for haemophilia will always be affected (display the phenotype)
Only sometimes will a female be affected if they have 1 copy of the mutation, however having 2 copies of the mutation (homozygous) will definitely result in the phenotype being displayed
How do the phenotypes of Haemophilia A and B compare?
They are identical
What is the occurence rate for Haemophilia A and B?
Haemophilia A - 1 in 10,000
Haemophilia B - 1 in 60,000
The residual level of which clotting hormones controls the severity of the haemophilia?
Coagulation Factor
A clotting factor concentration of <1% will result in what severity of haemophilia?
Severe
A clotting factor concentration of 1-5% will result in what severity of haemophilia?
Moderate
A clotting factor concentration of 5-30% will result in what severity of haemophilia?
Mild
What are the clinical features of haemophilia?
Haemarthrosis - Bleeding into joints (usually hing joints)
Muscle Haematoma
CNS bleeding
Retroperitoneal bleeding
Post surgical bleeding
What are the clinical complications of haemophilia?
1 - Synovitis
2 - Chronic haemophiliac arthropathy
3 - Neurovascular compression
4 - Stroke
How is haemophilia diagnosed?
1 - Clinical picture
2 - Prolonged APTT
3 - Normal PT
4 - Reduced Factor VIII or IX
If someone with haemophilia starts to bleed, how should they be treated?
Give them Factor VIII (A) or IX (B)
Apart from replacing the deficient coagulation factors, what are some other treatment options available for haemophilia bleeds?
Desmopressin
Tranexamic acid
What are the general treatment options for haemophilia?
Splints (to help joint pain)
Physio
Analgesia
Joint replacement
What are the common complications from treatment of haemophilia?
1) Viral infection from receiving blood - HIV, HBV, HCV
2) Antibodies to Factor VIII
3) DDAVP - MI’s
What is the occurence rate for von Willebrand Disease?
1 in 200
What type of bleeding occurs in vWD?
Platelet type bleeding (onto surfaces)
What causes vWD?
Abnormalities in vWF
